Abstract
We conducted a nation-wide survey to elucidate the characteristics of PSC as well as IgG4-SC without apparent pancreatic involvement diagnosed after 2005, by sending questionnaires. One-hundred and ninty-seven patients with PSC and 43 patients with IgG4-SC were identified. Compared to PSC, IgG4-SC was male-dominant and developed in older patients. There were two peaks in the age distribution of PSC patients, as demonstrated in the previous nation-wide surveys. The main location of involved bile ducts was both intra and extrahepatic in PSC and intrahepatic in IgG4-SC. Inflammatory bowel diseases was noticed in 68/197 (34%) of PSC, relatively low as shown in the previous surveys. Three-year survival rate were 85.0% in PSC and 90.0% in IgG4-SC, suggesting better prognosis of IgG4-SC.
